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Vol. 25. Num. 4.July - August 2014
Pages 149-198
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Vol. 25. Num. 4.July - August 2014
Pages 149-198
Case report
DOI: 10.1016/j.neucir.2014.01.001
Epithelioid sarcoma of the spine: Case report and literature review
Sarcoma epitelioide de la columna vertebral: caso clínico y revisión de la literatura
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Clara Chamadoiraa,??
Corresponding author
clarachamadoira@gmail.com

Corresponding author.
, Paulo Pereiraa, Pedro S. Silvaa, Ligia Castrob, Rui Vaza
a Servicio de Neurocirugía, Hospital São João, Porto. Portugal
b Servicio de Anatomía Patológica, Hospital São João, Porto. Portugal
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Abstract

Epithelioid sarcomas are rare mesenchymal neoplasms mainly arising in the limbs of young adults. We report the case of a 24-year-old male presenting low back pain radiating to both lower limbs, constipation and urinary retention. The MRI scan showed an intraspinal lesion extending from L4 to S2. Surgery resulted in gross total removal of the extradural lesion and partial removal of the intradural component. The immunohistological study of the lesion was consistent with an epithelioid sarcoma. The patient was submitted to radiotherapy and chemotherapy, but a local recurrence of the lesion and dissemination along the neuraxis were observed 3 months after surgery. Despite treatment, the patient died 4 months after the surgical procedure due to multiorgan failure.

Despite there being isolated reports of epithelioid sarcomas appearing in the spine, this is, to our knowledge, the first case with intradural extension.

Keywords:
Dura mater
Epithelioid sarcoma
Immunohistochemistry
Spine
Resumen

Los sarcomas epitelioides son neoplasias mesenquimatosas raras que afectan sobre todo a extremidades de adultos jóvenes. Presentamos el caso de un adulto de 24 años de edad y sexo masculino que consulta por lumbalgia irradiada a miembros inferiores, estreñimiento y retención urinaria. La RM mostró una lesión espinal que se extendía de L4 a S2. La cirugía consistió en una resección casi total de la lesión extradural y parcial del componente intradural.

El estudio inmunohistológico de la lesión fue compatible con un sarcoma epitelioide. El paciente fue sometido a radioterapia y quimioterapia, pero 3 meses después de la cirugía se verificó recurrencia local de la lesión y diseminación tumoral a lo largo del neuroeje. A pesar del tratamiento, el paciente falleció 4 meses después de la cirugía por fallo multiorgánico.

Este es el primer caso descrito en la literatura de sarcoma epitelioide espinal con extensión intradural.

Palabras clave:
Duramadre
Sarcoma epitelioide
Inmunohistoquímica
Columna vertebral

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